Brady Cunningham, the infant who helped spur Missouri legislators to require testing for Krabbe disease. He died in April 2009. / Submitted photo

Brady Cunningham, the infant who helped spur Missouri legislators to require testing for Krabbe disease. He died in April 2009. / Submitted photo

Brady Cunningham, the infant who helped spur Missouri legislators to require testing for Krabbe disease. He died in April 2009. / Submitted photo

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Blood samples of children born in Missouri are being shipped to a lab in New York to test for a rare genetic disorder that in its worst form can be fatal.

The disorder, called Krabbe disease, affects about 1 in 100,000 newborns. The only treatment is a bone marrow transplant or cord blood transplant, which can also be fatal.

“The New York State Health Laboratory began receiving samples on Aug. 30 and is now testing samples of all newborns dating back to Aug. 22 and after,” said Misti Preston, a spokeswoman for the Missouri Department of Health and Senior Services.

Missouri officials signed the one-year contract in August after St. Louis Cardinals player Skip Schumaker and team announcer Dan McLaughlin teamed up to promote awareness of the disease. McLaughlin mentioned during games that Missouri was not screening for the disease although the state has a law on the books saying that newborns would be screened.

“Skip is a good guy,” McLaughlin said. “He felt very passionate about this.”

Preston did not respond to a question about what role, if any, Schumaker’s involvement played in the state’s decision to start testing.

A federal advisory panel on newborn screenings doesn’t recommend routine screening for Krabbe because of the possibility of “substantial harm” in treatment for the disease or testing for it. The March of Dimes, the children’s health advocacy group, also doesn’t advocate that states require testing for the disease.

New York is the only other state that routinely tests infants for Krabbe. New York started its testing after lobbying by former Buffalo Bills quarterback Jim Kelly and his wife Jill, whose son died of the disease.

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A report done for the federal advisory panel in 2009 found that New York identified two newborns who met the criteria for the most severe form of the disease, early infantile Krabbe, after testing more than 769,800 newborns. Both babies underwent cord blood transplants in their first month of life. One died.

The report also said that children who underwent transplants and survived had poor brain growth and couldn’t walk on their own.

Alex Kemper, an associate professor at the Duke University School of Medicine, said Missouri needs to ensure that infants who are identified in screening are appropriately evaluated afterward. The disease has a range of severity, which includes damage to the central nervous system. It can also cause blindness and deafness.

“Because the treatment is a bone marrow transplant you don’t want to do a bone marrow transplant in someone who doesn’t have the early infantile form of the disease,” Kemper said.

Schumaker said he supports testing for Krabbe because of his experience with his friend’s daughter.

“As a parent, you would do everything you could to give your kid a fighting chance to live,” he said. “At the very least this bone marrow gives the parents hope that their kid might beat this horrible disease. Does it hurt worse than watching your kid die slowly in front of your eyes?”

Grandmother pushed for law

The grandmother of the Missouri infant whose name is on the Missouri law said Schumaker’s involvement helped spur the state to test for the disease. Brady Cunningham died in April 2009, 10 days before his first birthday.

“When you bring somebody like Skip Schumaker on board that has that professional sports personality that helps,” said Becky Ruth.

Brady’s parents, Dustin and Jessy Cunningham, said they were happy to see the testing begin.

“We lost our son,” said Dustin Cunningham. “All this is being done in honor of our son. We’re just proud parents.”

In May, the Cunningham family learned that the original Missouri contract didn’t include testing for Krabbe. They contacted Missouri officials about it but were frustrated with the response they received.

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“We are still receiving no response from the governor’s office,” Ruth said in July. “The attorney general’s office says it is not their jurisdiction.”

Missouri’s not testing for the disease also became an issue in the race for attorney general when Republican candidate Ed Martin, a friend of Ruth’s, put out a press release on the issue.

“When a family relies on the law being the law and they get told there’s nothing to be done it seems to me that’s worrying,” Martin said.

Nanci Gonder, a spokeswoman for Attorney General Chris Koster, said Koster’s staff contacted DHSS in early June about the testing.

“They told us they were working on a solution for it,” Gonder said.

Schumaker became involved because he has a childhood friend in Arizona, Sean Stutzman, whose daughter, Reesa, is dying from the disease.

She can’t smile, laugh, eat on her own, sit up or roll over.

“She’s got a really cute personality in her own way, but she can’t do much,” Stutzman said.

The Cunningham family went to a fundraiser for Reesa in Missouri and met Schumaker who asked what he could do to help.

“I was going to get involved with the governor if I need to, but it kind of grew legs on its own because of the awareness that was created by the Cardinals community and Fox Network and radio shows,” Shumaker told the web site stlsportspage.com.

Stutzman believes in the importance of screening all babies for Krabbe and is glad his friend Schumaker was able to help in Missouri. Stutzman’s family is talking about trying to get Arizona to also require screening for Krabbe.

The Stutzman family maintains a web site about Baby Reesa. Stutzman said the family plans to have a baby naming ceremony, a Jewish tradition, for the infant. They don’t know how much longer she might live.

“The influence of a sports figure just brought to light a bigger issue that the law that was passed is not being enforced,” Stutzman said. “Influence. That’s the way the world works.”